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Past and current cause-specific mortality in Eisenmenger syndrome.
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2017 (English)In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 38, no 26, p. 2060-2067Article in journal (Refereed) Published
Abstract [en]

Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015.

Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001).

Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

Place, publisher, year, edition, pages
2017. Vol. 38, no 26, p. 2060-2067
Keywords [en]
Cause of death, Eisenmenger syndrome, Heart failure, Mortality, Pulmonary arterial hypertension
National Category
Cardiac and Cardiovascular Systems
Research subject
Cardiology
Identifiers
URN: urn:nbn:se:umu:diva-146411DOI: 10.1093/eurheartj/ehx201PubMedID: 28430906OAI: oai:DiVA.org:umu-146411DiVA, id: diva2:1196407
Available from: 2018-04-09 Created: 2018-04-09 Last updated: 2018-04-09

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